whom 3 cases had the limbic system involvement, and 2 cases were mainly had extra limbic area involvement. Three cases had hippocampal atrophy or sclerosis during follow-up. All 8 patients were treated with immunotherapy. After immunotherapy, all patients had short-term improvement. Follow-up for 6 months to 6 years showed that 3 cases with extra limbic encephalitis improved to baseline levels, and 5 limbic encephalitis cases had poor outcomes, including 1 death and 4 cases still had focal epilepsy. Conclusions Pediatric anti-GAD65 antibody associated autoimmune encephalitis is a rare but treatable disease, including limbic encephalitis and extra limbic encephalitis. The most common clinical manifestations are seizures and memory impairment. Early diagnosis and immunotherapy can improve the symptoms in a short time. But patients with limbic encephalitis often had refractory epilepsy in the chronic phase, and have a poor long-term outcome.Objective To investigate the clinical characteristics and genetic features of congenital myasthenic syndrome (CMS) related to SLC25A1 gene variant. Methods The clinical data of two SLC25A1 gene variant related CMS patients treated at the Children's Hospital of Fudan University between January 2015 and June 2019 were analyzed retrospectively. A literature search with "SLC25A1" and "congenital myasthenic syndrome" as key words was conducted at China National Knowledge Infrastructure, Wanfang Data Knowledge Service Platform, National Center from Biotechnology Information and Pubmed (up to June 2020). The clinical characteristics and genetic features of congenital myasthenic syndrome related to SLC25A1 gene variant were summarized. Results Two patients were all males, aged 9 years and 2 years respectively and the onset age was in infancy. In addition to typical CMS symptoms (fatigable muscular weakness, including bilateral ptosis, strabismus, masticatory weakness, low voice and limb weakness), the two patients botual disability or developmental delay. Part of patients had metabolic abnormalities. The variants (c.740G&gt;A, p.R247Q and c.145G&gt;A, p.V49M) are recurrent.Objective To analyze the clinical characteristics and long-term outcome of Langerhans cell histiocytosis with multisystem involvement (MS-LCH) in children, and to evaluate the efficacy of modified DAL-HX83/90 protocol. Methods This retrospective study included 53 patients with MS-LCH admitted to the Department of Pediatric Hematology and Oncology, First Affiliated Hospital of Zhengzhou University from January 2011 to May 2019. Modified DAL-HX83/90 protocol was used in all patients as an initial treatment. The patients were divided into the group with (RO+) or without (RO-) risk organ involvement. The RO+group was further divided into two groups, as RO+? group (lung involvement only) and RO+? group (extra-pulmonary, with or without lung involvement). The clinical characteristics and the long-term outcome were summarized. Event-free survival (EFS) and overall survival (OS) curves were analyzed with Kaplan-Meier method. Univariate and multivariate analysis of prognostic factors including age, sex, risk organ inv). Conclusions Most of the children without risk organ involvement treated with modified DAL-HX83/90 protocol could achieve long-term survival. However, the children involved liver, spleen, or hematopoietic system had a high risk of disease progression and recurrence.Objective To summarize the clinical characteristics, imaging features, diagnosis, treatment and prognosis of pulmonary actinomycosis in children. Methods The clinical data of a child with pulmonary actinomycosis who was hospitalized in Children's Hospital, Zhejiang University School of Medicine in December 2019 was retrospectively analyzed. The related literature published from January 1975 to January 2020 was retrieved from Wanfang, CNKI and PubMed databases with "pulmonary" or "thoracic" and "actinomycosis" and "pediatric" or "children" or "child" as the keywords. And the characteristics of pediatric pulmonary actinomycosis were summarized based on the literature review. Results The patient was a boy aged 12 years and 6 months. He was admitted due to cough and chest pain for more than 20 days, with fever on the first three days. The chest CT scan in local hospital found inflammatory lesions in the right middle lobe, which was also suspected to be cavitation. The flexible bronchoscopy showed congestion and eses (8 cases), cough (23 cases), pain (chest, back, shoulders and armpits) (24 cases), fever (25 cases), weight loss (26 cases), etc. https://www.selleckchem.com/products/nx-5948.html Conclusions The clinical manifestations and imaging features of pediatric pulmonary actinomycosis are nonspecific, therefore it could easily be misdiagnosed. For children with pneumonia of unknown etiology and failing to respond to routine antibiotics, the pathogen metagene sequencing of the bronchoalveolar lavage fluid will be helpful for diagnosis. With appropriate course of antibiotic treatment, the prognosis is good in most cases.Objective To summarize the clinical manifestations and to evaluate the feasibility of therapeutic bronchoscopy in pediatric primary airway tumors. Methods The clinical data of 8 children presented with airway tumors in Beijing Children's Hospital from November 2016 to March 2018 were retrospectively analyzed. Descriptive analysis was used to summarize the outcome and prognosis of these children after interventional bronchoscopy. Results Among the 8 children, 5 were male and 3 were female, aged from 4 years and 8 months to 9 years and 2 months. The main clinical manifestations were cough and wheezing in 6 cases, hemoptysis in 3 cases and recurrent pulmonary infection in 2 cases, and none of them had lymph node metastasis and extra-pulmonary metastasis. All 8 children underwent bronchoscopic resection of the tumors, and the 4 children with blood-rich tumors had bronchial artery embolization at the blood supply site before the resection. No major bleeding or pneumothorax occurred during or after the operation. Four cases had low grade malignant bronchial mucoepidermoid carcinoma, 2 had inflammatory myofibroblastoma, and the rest two had pleomorphic adenoma and bronchial smooth muscle spindle cell tumor. Postoperative follow-up lasted 2 to 4 years, and 3 had tumor recured. Among the 3 cases, 2 had inflammatory myofibroblastoma who underwent surgical resection of the lesion and combined chemotherapy, respectively; and one had mucoepidermoid carcinoma who was under follow-up after interventional cryotherapy. Conclusions The clinical manifestations of primary airway tumors in children are atypical. The main clinical manifestations are dry cough, wheezing, recurrent pulmonary infection and hemoptysis. Interventional bronchoscopy is a feasible choice for the treatment of pediatric airway tumors.